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Criteria for the Classification of Systemic Sclerosis (Scleroderma)

1980 Criteria for the Classification of Systemic Sclerosis

The American College of Rheumatology (former American Rheumatism Association - ARA) has defined criteria, that are 97 % sensitive and 98 % specific for systemic sclerosis (SSc) as follows:

Major criterion:

  • Proximal diffuse (truncal) sclerosis (skin tightness, thickening, non-pitting induration)

Minor criteria:

  • Sclerodactyly (only fingers and/or toes)

  • Digital pitting scars or loss of substance of the digital finger pads (pulp loss)

  • Bilateral basilar pulmonary fibrosis

The patient should fulfill the major criterion or two of the three minor criteria. Raynaud's phenomenon is observed in 90-98 % of SSc patients.

Subsets of Systemic Sclerosis




Skin involvement

Distal and proximal

extremities, face, trunk

Distal to elbows, face

Raynaud’s phenomenon

Onset within 1 year or at time of skin changes

May precede skin disease by years

Organ involvement

Pulmonary (interstitial fibrosis); renal (renovascular hypertensive crisis); gastrointestinal; cardiac

Gastrointestinal; pulmonary arterial hypertension after 10-15 years of disease in <10% of patients; biliary cirrhosis

Nail fold capillaries

Dilatation and dropout

Dilatation without significant dropout

Antinuclear antibodies

Anti-topoisomerase 1


* Also referred to as CREST( calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasia).


ABCDCREST Criteria for the Classification of Systemic Sclerosis

  1. Autoantibodies: autoantibodies to centromere proteins (CENPs) detected by indirect immunofluorescence; anti-Scl-70 (topoisomerase I) detected by double immunodiffusion; anti-fibrillarin (U3-RNP) detected by immunoprecipitation

  2. Bibasilar pulmonary fibrosis detected by chest radiograph: linear shadows or “honey-comb” reticular appearance most expressed at the periphery of the lungs and at the bases

  3. Contracture of the joints defined as permanent limitation of joint motion. The prayer sign is detected when a patient opposed the palmar surfaces of both hands with extended wrists. The sign is positive when the patient is unable to oppose the palms. This suggests joint or skin pathology, or shortening of the forearm flexors

  4. Dermal thickening can be defined by the modified Rodnan skin score, which employs clinical palpation of the skin as described

  5. Calcinosis cutis, most often located on the fingers, is intra and/or subcutaneous deposits of hydroxyapatite that can ulcerate the skin; it can be detected by radiography, crystallographic or chemical analysis

  6. Raynaud’s phenomenon is a sudden pallor of an acral structure (e.g., fingers, whole hand, toes, tip of nose, earlobe, or tongue). The involved area may subsequently develop cyanosis and, with re-warming, become erythematous. Determination is by patient’s history or physician’s observation

  7. Esophageal distal hypomotility can be detected by cine/video barium esophagram, performed in the upright and supine position. Reflux-esophagitis can be detected by esophagogastroduodenoscopy in the forms of erosive esophagitis or Barret’s esophagus

  8. Sclerodactyly is symmetric thickening and tightening of the skin on the digits. Before sclerodactyly develops there could be a phase of non-pitting digital edema of varying duration. It is defined as non-pitting increase in soft tissue mass of the digits that extends beyond the normal confines of the joint capsules

  9. Teleangiectasias are visible macular dilatations of superficial cutaneous blood vessels that collapse upon pressure and fill slowly when pressure is released. Common locations are the digits, face, lips, tongue

A classification of definite SSc requires three or more criteria.


Related Criteria

Classification Criteria for the Diagnosis of Systemic Lupus Erythematosus (SLE)

ACR Criteria for the Classification of Polyarteritis Nodosa (PAN)

ACR Criteria for the Classification of Churg-Strauss Syndrome (CSS)

ACR Criteria for the Classification of Giant-Cell Arteritis

ACR Criteria for the Classification of Hypersensitivity Vasculitis

Revised International Classification Criteria for Sjögren's Syndrome (SS)

1990 Criteria of American College of Rheumatology for the Classification of Takayasu Arteritis

Classification Criteria for Osteoarthritis

Diagnostic Criteria for Relapsing Polychondritis

Criteria for the Classification of Wegener's Granulomatosis (WG)

Diagnostic Criteria for Autoimmune Hepatitis (AIH)

Diagnostic Criteria for Kawasaki Disease

Names and definitions of vasculitis adopted by the Chapel Hill Consensus Conference

Diagnostic Criteria for Mixed Connective Tissue Disease (MCTD)

Criteria for the Classification of the Antiphospholipid Syndrome (APS)



  1. Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980;23:581-90. [Medline]

  2. Nadashkevich O, Davis P, Fritzler MJ. A proposal of criteria for the classification of systemic sclerosis. Med. Sci. Monit. 2004 Nov;10(11):CR615-21. Epub 2004 Oct 26. [Medline]

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Created: Jul 20, 2007
Last Modified: 10/24/2010

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